Abstract：
:Early onset dementia (EOD) is a major diagnostic challenge as it often presents with atypical features and may be attributed to treatable diseases. Primary degenerative dementias (Alzheimer's disease-AD, frontotemporal lobar degeneration-FTLD, Lewy body dementia-LBD), although traditionally considered to affect older people, are still a main cause of EOD. 491 demented patients were assessed from January 1, 2003 to December 31, 2010 in the Neurology Department of a tertiary referral center. Patients were classified as AD, behavioral variant frontotemporal dementia (bvFTD), non-fluent agrammatic variant primary progressive aphasia (naPPA), semantic variant PPA (svPPA), corticobasal degeneration (CBD), or progressive supranuclear palsy (PSP) who also met criteria for naPPA and LBD. Finally, their demographic characteristics were analysed, according to age at onset (EOD <65 years, late onset dementia-LOD ≥65 years). From the 491 patients, 137 (27.9 %) were EOD. In the EOD group, 52 (38 %) were diagnosed with bvFTD, 34 (24.8 %) with AD, 27 (19.7 %) with naPPA, 10 (7.2 %) with svPPA, 12 (8.8 %) with CBD or PSP, and 2 (1.5 %) with LBD. Demographic characteristics did not differ significantly among diagnostic categories in the EOD group, while in the LOD group FTLD patients were younger and more frequently men compared to both AD and LBD patients. EOD patients had more years of education than LOD patients. Degenerative disorders as causes of EOD are not rare. High clinical alertness is warranted to achieve correct and timely diagnosis.
journal_name
Acta Neurol Belgjournal_title
Acta neurologica Belgicaauthors
Maiovis P,Ioannidis P,Konstantinopoulou E,Karacostas Ddoi
10.1007/s13760-014-0310-2subject
Has Abstractpub_date
2015-03-01 00:00:00pages
27-31issue
1eissn
0300-9009issn
2240-2993journal_volume
115pub_type
杂志文章abstract：:Amyotrophic Lateral Sclerosis and CHARGE syndrome are complex neurological disorders, which never occurred together in the same family and, to date, no putative correlation between them has been described on PubMed Central. Due to our aim was to evaluate the presence of different genetic variants involved in these pat...
journal_title：Acta neurologica Belgica
pub_type： 杂志文章
doi：10.1007/s13760-018-1029-2
更新日期：2018-12-01 00:00:00
abstract：:Visual disorder is one of the non-motor symptoms found in Parkinson's disease (PD). It can be easily identified in the early stages even before the spread of pathological conditions to the brain parts. Studies have revealed that loss of dopamine (DA) cells in retinal layers is a prime cause for both retinal disturbanc...
journal_title：Acta neurologica Belgica
pub_type： 杂志文章,评审
doi：10.1007/s13760-020-01274-w
更新日期：2020-04-01 00:00:00
abstract：:For the most part, the central nervous system is unable to regenerate. The majority of injuries of vascular, inflammatory, degenerative and traumatic aetiology lead to an irreversible loss of central nervous system function. The paper presents the role of Nogo-A, MAG and OMgp proteins in the inhibition of central nerv...
journal_title：Acta neurologica Belgica
pub_type： 杂志文章,评审
doi：10.1007/s13760-015-0425-0
更新日期：2015-12-01 00:00:00
abstract：:n-Hexane gives cause to one of the most common toxic polyneuropathies seen in poorly ventilated factories. It is a sensory-motor polyneuropathy ending up with axonal degeneration. Nerve biopsy reveals paranodal axonal swelling and secondary myelin retraction in early stages. Myelin retraction imitates demyelination ca...
journal_title：Acta neurologica Belgica
pub_type： 杂志文章
doi：10.1007/s13760-019-01137-z
更新日期：2019-04-13 00:00:00
abstract：:Self-reporting scales are commonly utilized in determining appropriate treatment strategies and follow-up in hand-related disorders. Responsiveness is described as the ability of a scale to detect clinically significant changes. We aimed to evaluate responsiveness of Michigan Hand Outcomes Questionnaire (MHQ), Duruöz ...
journal_title：Acta neurologica Belgica
pub_type： 杂志文章
doi：10.1007/s13760-020-01443-x
更新日期：2020-07-15 00:00:00
abstract：:A 54-year-old female patient presented acute hallucinatory psychotic episodes with irritative EEG for seven years before an initial state of complex partial mal. A single convulsive episode occurred 14 years after the beginning of the psychiatric symptoms. The intercritical EEG showed independent temporal lobe foci pr...
journal_title：Acta neurologica Belgica
pub_type： 杂志文章
doi：
更新日期：1989-01-01 00:00:00
abstract：: ...
journal_title：Acta neurologica Belgica
pub_type：
doi：10.1007/s13760-018-1035-4
更新日期：2018-12-01 00:00:00
abstract：:Electrophysiological tests (visual and somatosensory evoked potentials, flicker fusion test) and electrophoretic examination of cerebrospinal fluid were performed in 17 patients who previously suffered from one or more attacks of optic neuritis. The association of the tests revealed the demyelinating inflammatory path...
journal_title：Acta neurologica Belgica
pub_type： 杂志文章
doi：
更新日期：1983-03-01 00:00:00
abstract：:Epilepsy is a neurological disorder consisting of recurrent seizures, resulting from excessive, uncontrolled electrical activity in the brain. Epilepsy treatment is successful in the majority of the cases; however; still one third of the epilepsy patients are refractory to treatment. Besides the ongoing research on th...
journal_title：Acta neurologica Belgica
pub_type： 杂志文章,评审
doi：
更新日期：2006-06-01 00:00:00
abstract：:Smoking is associated with increased multiple sclerosis (MS) risk. In addition, some studies have reported that smoking is associated with anxiety and depression. However, the associations between smoking, walking, and fatigue are needed to be investigated. The objective was to investigate the associations between cig...
journal_title：Acta neurologica Belgica
pub_type： 杂志文章
doi：10.1007/s13760-020-01341-2
更新日期：2020-03-28 00:00:00
abstract：:The gastrocnemius and quadriceps muscles findings in 18 patients with chronic arterial insufficiency were reviewed with regard to mitochondrial changes. Prominent mitochondrial alterations were present in eight out of 18 patients. The comparison of clinical data between patients with and without mitochondrial changes ...
journal_title：Acta neurologica Belgica
pub_type： 杂志文章
doi：
更新日期：1986-11-01 00:00:00
abstract：:The authors report the case of a 26 year old woman with acute anterior poliomyelitis contracted during the vaccination of her baby. Despite having been herself vaccinated in infancy she was not protected against the poliovirus. The clinical interest of this uncommon case is a severe paralytic state with definitive par...
journal_title：Acta neurologica Belgica
pub_type： 杂志文章
doi：
更新日期：1989-11-01 00:00:00
abstract：:BRAT1-related neurodevelopmental disorders are characterized by heterogeneous phenotypes with varying levels of clinical severity. Since the discovery of BRAT1 variants as the molecular etiology of lethal neonatal rigidity and multifocal seizure syndrome (RMFSL, OMIM 614498), these variants have also been identified i...
journal_title：Acta neurologica Belgica
pub_type： 杂志文章
doi：10.1007/s13760-020-01513-0
更新日期：2020-12-01 00:00:00
abstract：:The various neurological manifestations encountered in 40 zairians out of a total of 500 with homozygous (SS) Sickle cell disease were described. These included convulsions, vascular accidents, mental changes, increased intracranial pressure and meningism. The literature on the involvement of the nervous system in Sic...
journal_title：Acta neurologica Belgica
pub_type： 杂志文章
doi：
更新日期：1977-03-01 00:00:00
abstract：:Between January 1992 and June 1995, 160 patients were presurgically evaluated for medically refractory epilepsy by the Epilepsy Monitoring and Surgery Team at the University Hospital of Gent. All these patients underwent a comprehensive presurgical evaluation, including extensive neurological history and examination, ...
journal_title：Acta neurologica Belgica
pub_type： 杂志文章
doi：
更新日期：1996-03-01 00:00:00
abstract：:Guillain-Barré Syndrome (GBS) is best viewed as a clinical syndrome which can have at least two clinical substrates: the commonest is an acquired demyelinating polyradiculoneuropathy and the other is an acute motor axonal neuropathy. An acute acquired neuropathy can also sometimes cause the Miller Fisher syndrome of o...
journal_title：Acta neurologica Belgica
pub_type： 杂志文章,评审
doi：
更新日期：1994-01-01 00:00:00
abstract：:This review focusses on the validation and standardization of Alzheimer's disease (AD) cerebrospinal fluid (CSF) biomarkers, as well as on the current clinical indications and rational use of CSF biomarkers in daily clinical practice. The validated AD CSF biomarkers, Aβ1-42, T-tau, and P-tau181, have an added value in...
journal_title：Acta neurologica Belgica
pub_type： 杂志文章,评审
doi：10.1007/s13760-017-0816-5
更新日期：2017-09-01 00:00:00
abstract：:The primary source of malignant intracerebral nerve sheath tumors is still unclear We report the imaging and MR spectroscopic findings in a 39-year-old man with a very rare brain stem tumor MR examination revealed the presence of intraaxial brain stem tumor with a partial exophytic growth. On pathological examination,...
journal_title：Acta neurologica Belgica
pub_type： 杂志文章
doi：
更新日期：2008-06-01 00:00:00
abstract：:We report the case of a 44-year-old woman with chronic facial pain. She was treated with several analgesics, prophylactic medications and infiltrations, but all treatment modalities were ineffective. Finally, the diagnosis of medication-overuse headache complicating migraine without aura was made and an appropriate tr...
journal_title：Acta neurologica Belgica
pub_type： 杂志文章
doi：
更新日期：2009-09-01 00:00:00
abstract：:We performed a retrospective study of 62 patients with syringomyelia. Most noteworthy among the clinical features in our patient population are the following: segmental sensory disturbances are usually less restrictive than the classical thermoanalgesia, pain is a presenting symptom in almost 50% of cases, and skeleta...
journal_title：Acta neurologica Belgica
pub_type： 杂志文章
doi：
更新日期：1990-01-01 00:00:00
abstract：:Eight cases of acute or subacute hypercalcemic encephalopathy are described. The symptoms comprise digestive disorders, signs of dehydration and neuropsychological anomalies. Among the latter, it is stressed that impairments of upright stance and locomotion are present in all cases. The clinical picture is not charact...
journal_title：Acta neurologica Belgica
pub_type： 杂志文章
doi：
更新日期：1977-05-01 00:00:00
abstract：:Despite the huge health and economic burden of migraine headache, few medications have been approved for its prophylactic treatment, most of which can potentially induce serious adverse effects. Coenzyme Q10 (CoQ10) is a supplement and has shown preliminary benefits in migraine prophylaxis. We aimed to assess this eff...
journal_title：Acta neurologica Belgica
pub_type： 临床试验,杂志文章
doi：10.1007/s13760-016-0697-z
更新日期：2017-03-01 00:00:00
abstract：:Celiac disease (CD) and non celiac gluten sensitivity (NCGS) can be responsible for neurological complications such as ataxia and peripheral neuropathies but also cognitive impairment. This cognitive involvement is variable in its expression, its duration and its prognosis ranging from transient and reversible subtle ...
journal_title：Acta neurologica Belgica
pub_type： 杂志文章,评审
doi：10.1007/s13760-017-0870-z
更新日期：2018-03-01 00:00:00
abstract：:Among the last 7000 consecutive autopsies from our department, nine cases of primary reticulum cell sarcoma (non hodgkinian malignant lymphoma) of the central nervous system were discovered. Another case was found by cerebral biopsy. There were six males and four females (mean age 43.5 years). Mean duration of illness...
journal_title：Acta neurologica Belgica
pub_type： 杂志文章
doi：
更新日期：1976-07-01 00:00:00
abstract：:Chorea paralytica (or chorea mollis) is a very rare variant of Sydenham's chorea, characterized by a profound hypotonia, resulting in severe disability. Given the rarity of this condition, data on its prognosis are lacking. Most reports suggest that the delay from onset to recover total autonomy is long, usually sever...
journal_title：Acta neurologica Belgica
pub_type： 杂志文章
doi：10.1007/s13760-013-0214-6
更新日期：2013-12-01 00:00:00
abstract：:After a lapse of not less than two years the authors study the functional and clinical evolution of 62 patients presenting myelopathy in cervical arthrosis treated by surgery. A scoring system for evaluating the functional deficit of the patients is used for quantitative assessment of the disorders. Surgery brought a ...
journal_title：Acta neurologica Belgica
pub_type： 杂志文章
doi：
更新日期：1986-03-01 00:00:00
abstract：:We wanted to explore possible differences in disease presentation, frequency, and age of onset of dementia with Lewy bodies (DLB) between first-generation immigrants (FGI) and patients born in Belgium (PBIB). We conducted a retrospective study on all patients of our Memory Clinic between June 1, 2010 and January 31, 2...
journal_title：Acta neurologica Belgica
pub_type： 杂志文章
doi：10.1007/s13760-020-01492-2
更新日期：2020-09-15 00:00:00
abstract：:We report a consanguineous family with a homozygous and heterozygous membrane metallo-endopeptidase (MME) mutation (c.467delC) and two clinical conditions: fetomaternal alloimmune membranous glomerulopathy (FMG) and hereditary motor and sensory axonal neuropathy. The penetrance of both phenotypes was variable. Some in...
journal_title：Acta neurologica Belgica
pub_type： 杂志文章
doi：10.1007/s13760-020-01275-9
更新日期：2020-02-01 00:00:00
abstract：:A case of isolated cerebral vasculitis showing benign outcome is presented. Review of pertinent literature suggests that isolated benign arteritis of the CNS should be considered as a distinct entity, in a heterogeneous group of vasculitides. The common features of the syndrome are as follows: 1(0) benign evolution; 2...
journal_title：Acta neurologica Belgica
pub_type： 杂志文章
doi：
更新日期：1984-08-01 00:00:00
abstract：:The authors report the case of a 17 year-old female patient who presented acute ascending polyneuritis of the Landry-Guillain-Barré type extending to all cranial nerve pairs during the abatement of hepatitis of unspecified etiology. The patient remained in a state of coma vigil for five days prior to gradual but full ...
journal_title：Acta neurologica Belgica
pub_type： 杂志文章
doi：
更新日期：1980-07-01 00:00:00
